Budd-Chiari syndrome: symptoms, life expectancy, frequency

Budd-Chiari syndrome can be dangerous if left untreated. What symptoms indicate liver disease and what it means for life expectancy.

Budd-Chiari syndrome is a rare liver disease in which blood accumulates in the liver because the hepatic veins or inferior vena cava are blocked.

Normally, blood flows from the liver through several veins into the vena cava, which then carries it to the heart. However, if one or more of these veins are blocked, such as by a blood clot, blood flow is restricted. Then more and more blood collects in the liver, which can cause numerous problems. In the worst case, they can reach life-threatening proportions. To prevent this, prompt treatment is important.

Those who suffer from Budd-Chiari syndrome do not always notice it immediately – some of those affected experience no symptoms or only mild symptoms. But they are in the minority.

As a rule, Budd-Chiari syndrome causes clearly noticeable and sometimes stressful symptoms, primarily the following:

• stomach pain
• a palpable enlargement of the liver
• an increased abdominal circumference

The liver swells because more and more blood is building up in it. This means the organ can also be sensitive to pressure.

The abdominal circumference increases because fluid collects in the abdominal cavity. This in turn is a result of blood congestion in the liver: the pressure in the blood vessels of the liver eventually becomes so high that fluid escapes from them into the abdominal cavity.

In addition to these symptoms typical of Budd-Chiari syndrome, other symptoms sometimes occur. These include, for example:

• Nausea, feeling of fullness and loss of appetite
• Fatigue and reduced resilience
• Yellowing of the skin (jaundice)
• bloody vomiting due to bleeding from varicose veins in the esophagus

However, none of the symptoms mentioned are specific to Budd-Chiari syndrome. So they can also have other causes – this is actually quite likely in Germany: Budd-Chiari syndrome is a rare disease in this country.

Budd-Chiari syndrome is also rare in other Western countries. It is estimated that approximately one in 2.5 million people in the Western population develop Budd-Chiari syndrome each year. However, the syndrome occurs more frequently in countries where living conditions are worse.

In principle, the disease can begin at any age. However, adults between the ages of 30 and 50 are often affected.

In many cases, Budd-Chiari syndrome occurs due to blood clots blocking the liver veins. This can occur, among other things, in various diseases in which the blood clots more easily than it should, or which affect the condition of the blood in other ways.

One of the following diseases or influences can often be identified in people with Budd-Chiari syndrome:

• genetically determined malignant diseases in which the blood-forming cells in the bone marrow multiply abnormally (myeloproliferative neoplasms)
• hereditary coagulation disorder (e.g. due to a congenital deficiency of protein C, protein S, antithrombin III or a factor V Leiden mutation)
• Taking birth control pills
• pregnancy
• chronic inflammatory bowel disease

Less commonly, the syndrome occurs when one or more liver veins are narrowed, meaning blood flow is impaired from the outside – for example by a tumor that puts pressure on the vein.

If detected early, Budd-Chiari syndrome is usually easy to treat. Therapy often consists of several measures. Among other things, medications are used that are intended to dissolve blood clots, so-called anticoagulants. If these are not enough, there are other therapeutic options to restore blood flow. One option, for example, is a surgical procedure in which an artificial connection (a so-called shunt) is created between the portal vein and the hepatic vein.

Without treatment, Budd-Chiari syndrome usually leads to death within a few months to three years. Patients who seek treatment in a timely manner have a significantly longer life expectancy: around 90 out of 100 affected people are still alive five years after diagnosis.

In principle, however, life expectancy also depends on which disease led to the blockage of the hepatic vein and how well it can be brought under control.